Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

被引:5
作者
Kavallar, Anna M. [1 ]
Messner, Franka [2 ]
Scheidl, Stefan [2 ]
Oberhuber, Rupert [2 ]
Schneeberger, Stefan [2 ]
Aldrian, Denise [1 ]
Berchtold, Valeria [2 ]
Sanal, Murat [2 ]
Entenmann, Andreas [1 ]
Straub, Simon [1 ]
Gasser, Anna [1 ]
Janecke, Andreas R. [1 ,3 ]
Mueller, Thomas [1 ]
Vogel, Georg F. [1 ,4 ]
机构
[1] Med Univ Innsbruck, Dept Paediat 1, A-6020 Innsbruck, Austria
[2] Med Univ Innsbruck, Dept Visceral Transplant & Thorac Surg, A-6020 Innsbruck, Austria
[3] Med Univ Innsbruck, Inst Human Genet, A-6020 Innsbruck, Austria
[4] Med Univ Innsbruck, Bioctr, Inst Cell Biol, A-6020 Innsbruck, Austria
来源
CHILDREN-BASEL | 2022年 / 9卷 / 12期
关键词
PFIC1; pediatric liver transplantation; steatosis; surgical biliary diversion; EXTERNAL BILIARY DIVERSION; SURGICAL-MANAGEMENT; BYLERS-DISEASE; ATP8B1; DIARRHEA; OUTCOMES; GENE;
D O I
10.3390/children9121964
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Background: Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the enterohepatic circulation, e.g., surgical biliary diversion (SBD) can slow down development of liver cirrhosis. Eventually, end stage liver disease necessitates liver transplantation (LT). PFIC1 patients might develop diarrhea, graft steatosis and inflammation after LT. SBD after LT was shown to be effective in the alleviation of liver steatosis and graft injury. Case report: Three PFIC1 patients received LT at the ages of two, two and a half and five years. Shortly after LT diarrhea and graft steatosis was recognized, SBD to the terminal ileum was opted to prevent risk for ascending cholangitis. After SBD, inflammation and steatosis was found to be reduced to resolved, as seen by liver biochemistry and ultrasounds. Diarrhea was reported unchanged. Conclusion: We present three PFIC1 cases for whom SBD to the terminal ileum successfully helped to resolve graft inflammation and steatosis.
引用
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页数:10
相关论文
共 32 条
[11]   Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease) [J].
Emond, JC ;
Whitington, PF .
JOURNAL OF PEDIATRIC SURGERY, 1995, 30 (12) :1635-1641
[12]   Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis [J].
Hang, Chenyue ;
Jin, Yijie ;
Luo, Yi ;
Feng, Mingxuan ;
Zhou, Tao ;
Zhu, Jianjun ;
Zhang, Jianjun ;
Liu, Yuan ;
Xia, Qiang .
JOURNAL OF CLINICAL MEDICINE, 2022, 11 (16)
[13]   Long-term liver transplant outcomes for progressive familial intrahepatic cholestasis type 1: The Pittsburgh experience [J].
Henkel, Sarah A. F. ;
Salgado, Claudia M. ;
Reyes-Mugica, Miguel ;
Soltys, Kyle A. ;
Strauss, Kevin ;
Mazariegos, George V. ;
Squires, Robert H. ;
McKiernan, Patrick J. ;
Zhang, Xingyu ;
Squires, James E. .
PEDIATRIC TRANSPLANTATION, 2021, 25 (08)
[14]   Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus [J].
Hollands, CM ;
Rivera-Pedrogo, FJ ;
Gonzalez-Vallina, R ;
Loret-de-Mola, O ;
Nahmad, M ;
Burnweit, CA .
JOURNAL OF PEDIATRIC SURGERY, 1998, 33 (02) :220-223
[15]  
Ismail H, 1999, Pediatr Transplant, V3, P219, DOI 10.1034/j.1399-3046.1999.00046.x
[16]   A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant [J].
Jankowska, Irena ;
Pawlowska, Joanna ;
Szymczak, Marek ;
Ismail, Hor ;
Broniszczak, Dorota ;
Cielecka-Kuszyk, Joanna ;
Socha, Piotr ;
Jarzebicka, Dorota ;
Czubkowski, Piotr .
AMERICAN JOURNAL OF CASE REPORTS, 2021, 22
[17]   Ileal bile acid transporter inhibition as an anticholestatic therapeutic target in biliary atresia and other cholestatic disorders [J].
Karpen, Saul J. ;
Kelly, Deirdre ;
Mack, Cara ;
Stein, Philip .
HEPATOLOGY INTERNATIONAL, 2020, 14 (05) :677-689
[18]   Liver Steatosis and Diarrhea After Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Can Biliary Diversion Solve These Problems? [J].
Knisely, A. S. ;
Houwen, Roderick H. J. .
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2021, 72 (03) :341-342
[19]   Surgical diversion of enterohepatic circulation in pediatric cholestasis [J].
Lemoine, Caroline ;
Superina, Riccardo .
SEMINARS IN PEDIATRIC SURGERY, 2020, 29 (04)
[20]   Hypothyroidism Associated with ATP8B1 Deficiency [J].
Li, Liting ;
Deheragoda, Maesha ;
Lu, Yi ;
Gong, Jingyu ;
Wang, Jianshe .
JOURNAL OF PEDIATRICS, 2015, 167 (06) :1334-+